Consensus statement from the first international colloquium on basal cell nevus syndrome (BCNS)

• Published in: American journal of medical genetics. Part A Vol. 155A; no. 9; pp. 2091 - 2097
• Main Authors: Bree, Alanna F., Shah, Maulik R.
• Format: Journal Article Conference Proceeding
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.09.2011; Wiley
• Subjects: Advisory Committees; basal cell carcinoma; basal cell carcinoma nevus syndrome; basal cell nevus syndrome; Basal Cell Nevus Syndrome - diagnosis; Basal Cell Nevus Syndrome - prevention & control; Basal Cell Nevus Syndrome - therapy; Basal cells; Carcinoma; Carcinoma, Basal Cell; Chromosome Disorders; Conferences; developmental bone diseases; Economics and Finance; genodermatoses; Gorlin syndrome; Gorlin-Goltz syndrome; hereditary neoplastic syndromes; Humanities and Social Sciences; Humans; Jaw Neoplasms; Medulloblastoma; Morbidity; Mortality; nevoid basal cell carcinoma syndrome; Nevus; odontogenic cysts; Pediatrics; primitive neuroectodermal tumor; Skin Neoplasms
• ISSN: 1552-4825; 1552-4833; 1552-4833
• DOI: 10.1002/ajmg.a.34128

The first international colloquium on basal cell nevus syndrome (BCNS) was held at Saint Louis University School of Medicine and supported by the Basal Cell Carcinoma Nevus Syndrome (BCCNS) Life Support Network (www.gorlinsyndrome.org). The foremost goal of the conference was to review and revise the prior diagnostic criteria and define the surveillance recommendations for affected pediatric and adult patients to allow for early intervention. The invited consensus group participants included geneticists, dermatologists, orthopedists, neurologists, and dental/oral medicine specialists, who treat patients with BCNS or related disorders. This group also included individuals who have a research interest in BCNS and who additionally serve on the medical advisory board of the BCCNS Life Support Network. Expert opinion was based on the collective clinical and research experience of the consensus group participants after presentation and review of the previously published literature regarding diagnosis and treatment of BCNS. A consensus was achieved and agreed upon by open roundtable discussion of the group participants. The consensus statement outlines the proposed diagnostic and management protocols that will hopefully limit morbidity and mortality for affected individuals until more specific and targeted therapies are widely available. © 2011 Wiley‐Liss, Inc.